D. Bron MD, PhD

New perspectives with PI3K inhibitors in B-cell malignant hemopathies

summary

Phosphoinositide 3-kinase inhibitors represent a new group of promising targeted therapies for malignant hemopathies and primarily lymphoproliferative disorders. This short report summarises recent knowledge on the mechanism of action, the rationale to use it in humans bearing malignant hemopathies and preliminary clinical trials’ data that led to the Food and Drug Administration approval of one of these compounds (idelalisib).

(BELG J HEMATOL 2015;6(4): 152–5)

Waldenstrom’s macroglobulinaemia: Belgian Hematology Society guidelines

summary

Waldenström’s macroglobulinaemia is a B-cell disorder characterised by bone marrow infiltration with lymphoplasmacytic cells, along with demonstration of an IgM monoclonal gammopathy in the blood. This condition belongs to the lymphoplasmacytic lymphomas as defined by the World Health Organization classification (ICD-0 code 9671/3). Approximately one-fourth of patients are asymptomatic. Clinical features of the symptomatic patients are diverse and may relate to overall disease burden (such as peripheral blood cytopaenias, organomegaly and constitutional symptoms) or may be directly attributable to the IgM paraprotein. The latter include hyperviscosity syndrome, amyloidosis, peripheral neuropathy and cold haemagglutinin. Therapeutic options have traditionally involved alkylating agents, nucleoside analogues, and rituximab, either as single therapy or in combination. However, emerging new data on combination therapy as well as novel agents have shown encouraging results. This report provides the Belgian Hematology Society guidelines according to recent clinical studies.

(BELG J HEMATOL 2015;6(4):142–50)

Highlights of the EHA

11 – 14 June 2015, Vienna, Austria
 

summary

2015 was an important year for the EHA, celebrating its 20^th anniversary, presenting ‘innovation in haematology’ as the theme of the year and welcoming almost 10.000 participants. Innovations were observed in many different diseases and selected major breakthroughs are summarised below.

(BELG J HEMATOL 2015;6(4): 169–72)

BHS Guidelines for the treatment of Burkitt’s lymphoma

Summary

Burkitt’s lymphoma is a rare but very aggressive non-Hodgkin’s lymphoma characterised by an isolated translocation t(8;14)(q24;q32). The sporadic form is the sub-entity most frequently encountered in Belgium. Diagnosis and initial work-up must be completed rapidly to start treatment as soon as possible. Positron emission tomography scan is useful for initial staging and to evaluate the chemosensitivity of the tumour during and after treatment. After debulking, it is recommended to add rituximab to chemotherapy. Currently intensive short-cycle and low intensity chemotherapies are two valuable options. Radiotherapy is not indicated except in case of central nervous system involvement. Patients achieving complete remission must be followed carefully during the first year to detect recurrence of the disease. More than 80% of patients sustain their remission one year following initial treatment and are considered cured. For patients in partial remission or with chemosensitive relapse, autologous stem cell transplantation is recommended following re-induction with non-cross-resistant polychemotherapy. Monitoring complete blood counts and cognitive functions is important to detect late toxicity of the applied therapies.

(BELG J HEMATOL 2015;6(2):61–9)

Challenges in the management of diffuse large B-cell lymphoma in older and much older patients

Introduction

Diffuse large B-cell lymphoma is a frequent pathology in older individuals, and though curable by R-CHOP 21, treatment toxicity increases in frail patients. Therefore, therapeutic choices have to take into account Comprehensive Geriatric Assessment in addition to Performance Status, but reliable and standardised clinical decision-making tools are sorely lacking. However, Mabthera-containing treatments adapted for frail patients and co-morbidities can be used with a satisfactory survival rate at two years. Nevertheless, the main cause of death remains disease progression.

(BELG J HEMATOL 2014;6(1):4–9)

P1.09 Follicular dendritic cell sarcoma in the retropharyngeal region: diagnostic and therapeutic challenges

P1.14 Monoclonal B-cell lymphocytosis (MBL): results of a routine screening in a general hospital